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Functional state following the Fontan procedure

Published online by Cambridge University Press:  01 August 2009

Ismee A. Williams ,
Lynn A. Sleeper ,
Steven D. Colan ,
Minmin Lu ,
Elizabeth A. Stephenson ,
Jane W. Newburger ,
Welton M. Gersony ,
Meryl S. Cohen ,
James F. Cnota  and
Andrew M. Atz
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Ismee A. Williams*
Affiliation:
Department of Pediatrics, Division of Cardiology, Columbia University Medical Centre, New York, New York, United States of America
Lynn A. Sleeper
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Steven D. Colan
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Minmin Lu
Affiliation:
New England Research Institutes, Watertown, Massachusetts, United States of America
Elizabeth A. Stephenson
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Canada
Jane W. Newburger
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Welton M. Gersony
Affiliation:
Department of Pediatrics, Division of Cardiology, Columbia University Medical Centre, New York, New York, United States of America
Meryl S. Cohen
Affiliation:
Department of Pediatrics, Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
James F. Cnota
Affiliation:
Department of Pediatric Cardiology, Wake Forest University School of Medicine, Winston-Salem, North Carolina, United States of America
Andrew M. Atz
Affiliation:
Department of Pediatrics, Division of Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America
Richard V. Williams
Affiliation:
Department of Pediatrics, Division of Cardiology, University of Utah, Salt Lake City, Utah, United States of America
Renee Margossian
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Andrew J. Powell
Affiliation:
Department of Cardiology, Children’s Hospital Boston, Boston, Massachusetts, United States of America
Mario P. Stylianou
Affiliation:
National Heart, Lung, and Blood Institute, Bethesda, MD, United States of America
Daphne T. Hsu
Affiliation:
Department of Pediatrics, Division of Cardiology, Columbia University Medical Centre, New York, New York, United States of America
*
Correspondence to: Ismee A. Williams, MD, MS, Columbia University College of Physicians & Surgeons, Morgan Stanley Children’s Hospital of New York, 2-North, 3959 Broadway, New York, NY 10032. Tel: (212) 342-1560; Fax: (212) 305-4429; E-mail: iib6@columbia.edu
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Abstract

Background

Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function.

Methods and Results

We analyzed data from 476 survivors with the Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset.

Conclusions

Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes.

Keywords

Clinical outcomessingle ventricleuniventricular heart
Type
Original Articles
Information
Cardiology in the Young , Volume 19 , Issue 4 , August 2009 , pp. 320 - 330
Copyright
Copyright © Cambridge University Press 2009

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